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Nursing Study Guide - Sickle Cell Anemia Cheat Sheet by

Nursing Study Guide for Sickle Cell Anemia in Pediatric Patients


Most common hemogl­obi­nop­athies
- RBCs do not carry the normal Hgb but instead carry a less effective type
Severe chronic blood disorder that occurs 1/2,000 per year in U.S
Glutamic acid is replaced w/ valine in Hgb molecule → elongated RBC that is rigid & sickle w/ a shortened life spa
Signif­icant anemia may occur when RBCs sickle
When cells sickle, blood becomes more viscous b/c cells clump together & prevent normal blood flow to the tissues of that area since their shape cannot pass through the smaller capill­aries & venules → vaso-o­ccl­usive process leads to local tissue hypoxia → ischemia → infarction & pain crisis
Hemolysis occurs following sickling

Etiology / Risk Factors

Inherited autosomal recessive pattern
African (1 in 400, 8% carry trait), Medite­rra­nean, Middle Eastern, & Indian descent
Passed on when both parents have gene or trait
May be triggered by stress or traumatic event
25% risk of Hgb SS, 25% Hgb AA, 50% Hgb AS
Infection, fever, acidosis, dehydr­ation, physical exertion, excessive cold exposure, hypoxia
Hgb AS = carrier & usually have only minimal health problems


Cardio­megaly, fxn murmur
Pulmonary HTN, Restri­ctive lung disease
Cholel­ith­iasis & gallstones
Jaundice, hepato­megaly
Functional Asplenia
Chronic leg ulcers
MODs common in adulthood
Delayed G&D & puberty

Signs & Symptoms

Infants asympt­omatic until 3-4 months d/t Hgb F protection (later half of 1st year of life)
Pain crisis, recurrent pain episodes (vaso-­occ­lusive)
- ↑ tachyc­ardia & tachypnea → more sickling
- Most common in joints (hot, swollen)
Acute Chest Syndrome (ACS)
- Cell clumping in lungs
- ↓ gas exchange → hypoxia, wheezing, cough, chest pain, fever → more sickling
Aplastic crisis (profound anemia)
Dactylitis (hand-foot syndrome), aseptic infarction
Pale mucous membranes
Easily fatigued w/ poor appetite
↓ BP d/t severe anemia or ↑ BP d/t SC nephro­pathy
Acute abdominal pain (most common) d/t sludging & spleno­megaly
Seques­tration crisis / Splenic seques­tration
- Pooling of blood in liver & spleen w/ ↓ blood volume & shock
Bacterial meningitis or sepsis
Bone infarction

Nursing Interv­entions

Immuni­zations & ABX to ↓ risk of infection
Tx underlying cause (infec­tion)
H-O-P to it! → Hydration, O2, Pain relief
O2 during episodes of crisis to prevent further sickling
↑ fluids to promote hemodi­lution, 150 mL/kg/day or double mainte­nance w/ hypotonic, D5W or D5 w/ 0.25% NS
Adequate pain management helps ↓ stress; always believe pain level
NO PRN pain meds, use fix dose, can use w/ non-ph­arm­aco­logic techniques
Assess pain w/ the right pain tool & look for compli­cations of pain
Assess for S/S of ineffe­ctive tissue perfusion
Avoid sudden temp change (cooling mattress for fever)
Cluster care
Quiet enviro­nment & privacy


Sickle­-Tu­rbidity Test (Sickl­edex) finger stick
Possib­ility or SCA or SC trait
Hgb Electr­oph­oresis
Dx, only accurate test for SCA
~6-9 mg/dL (normal 11-15 in infant)
Signif­icantly lower w/ splenic seques­tra­tion, ACS, or aplastic crisis
Reticu­locyte Count
↑ greatly
Peripheral Blood Smear
Presence of sickle­-shaped cells & target cells
Platelet Count, Erythr­ocyte Sedime­ntation Rate,
↑ bilirubin
X-Ray Studies or Scans
Determine extent of organ or tissue damage d/t vaso-o­ccl­usion
↑ d/t hemolysis of RBCs after transf­usion
Pulmonary Infiltrate

Collab­orative Care & Meds

Stem cell transplant
Prophy­lactic ABX
IV fluids


Family support as they often feel guilty or respon­sible
Promote wireless commun­ication w/ NP for collab & coaching
Disease process, compli­cat­ions, genetics, testing for carrier status
Regular health mainte­nance visits, immuni­zat­ions, PCN, coping, adequate fluids
Avoid temp fluctu­ations, overex­ertion, & stress
Need 24-hr access to facility that specia­lizes in SCA
Report & Seek Immediate Medical Attention:
- Suspected pain crisis
- Febrile illness
- Pale, listle­ssness, ↑ fatigue
- Unusual headache, loss of feeling, sudden weakness (stroke)
- Sudden vision changes
- Cough, SOB, chest pain (ACS)
- Limp or swollen joints
- Painful erection that won’t go down (priapism)
- Symmetric swelling of hands & feet in (dacty­litis)


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